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The Clinical Respiratory Journal Nov 2019Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been...
INTRODUCTION
Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH-CHD).
OBJECTIVES
The aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH-CHD patients during 12-month follow-up.
METHODS
We reviewed 288 Chinese PAH-CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low-risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow-up were collected. The association between low-risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed.
RESULTS
There were 105 PAH-CHD patients included in the final analysis. Twenty-nine patients had AEs defined as death, initiation of new or combined medication treatment, or re-hospitalisation because of the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6-minute walking distance (6MWD), NT-proBNP and SvO were significantly different between AE and AE-free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03-0.19, P < 0.001) and NT-proBNP (HR = 0.35, 95% CI: 0.16-0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low-risk criteria at diagnosis discriminated the risk of AEs (P < 0.001).
CONCLUSIONS
Among the low-risk criteria proposed by current guidelines, 6MWD and NT-proBNP predicted AEs independently for PAH-CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH-CHD patients.
Topics: Adult; China; Eisenmenger Complex; Female; Health Plan Implementation; Heart Defects, Congenital; Humans; Incidence; Infant; Male; Middle Aged; Natriuretic Peptide, Brain; Oxygen; Peptide Fragments; Practice Guidelines as Topic; Predictive Value of Tests; Prognosis; Pulmonary Arterial Hypertension; Retrospective Studies; Risk Assessment; Walk Test
PubMed: 31419027
DOI: 10.1111/crj.13076 -
Kidney & Blood Pressure Research 2024Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may... (Review)
Review
INTRODUCTION
Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES.
CASE PRESENTATION
A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength.
CONCLUSION
Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.
Topics: Humans; Female; Adult; Eisenmenger Complex; Kidney Diseases; Cyanosis; Polycythemia
PubMed: 38447536
DOI: 10.1159/000538100 -
Journal of Clinical Medicine May 2020Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional...
INTRODUCTION
Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension.
OBJECTIVE
To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data.
METHODS AND RESULTS
COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients´ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy ( = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status.
CONCLUSIONS
In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.
PubMed: 32414075
DOI: 10.3390/jcm9051456 -
Circulation. Cardiovascular Imaging Dec 2015Patients with Eisenmenger syndrome (ES) have better survival, despite similar pulmonary vascular pathology, compared with other patients with pulmonary arterial...
Impaired Right, Left, or Biventricular Function and Resting Oxygen Saturation Are Associated With Mortality in Eisenmenger Syndrome: A Clinical and Cardiovascular Magnetic Resonance Study.
BACKGROUND
Patients with Eisenmenger syndrome (ES) have better survival, despite similar pulmonary vascular pathology, compared with other patients with pulmonary arterial hypertension. Cardiovascular magnetic resonance (CMR) is useful for risk stratification in idiopathic pulmonary arterial hypertension, whereas it has not been evaluated in ES. We studied CMR together with other noninvasive measurements in ES to evaluate its potential role as a noninvasive risk stratification test.
METHODS AND RESULTS
Between 2003 and 2005, 48 patients with ES, all with a post-tricuspid shunt, were enrolled in a prospective, longitudinal, single-center study. All patients underwent a standardized baseline assessment with CMR, blood test, echocardiography, and 6-minute walk test and were followed up for mortality until the end of December 2013. Twelve patients (25%) died during follow-up, mostly from heart failure (50%). Impaired ventricular function (right or left ventricular ejection fraction) was associated with increased risk of mortality (lowest quartile: right ventricular ejection fraction, <40%; hazard ratio, 4.4 [95% confidence interval, 1.4-13.5]; P=0.01 and left ventricular ejection fraction, <50%; hazard ratio, 6.6 [95% confidence interval, 2.1-20.8]; P=0.001). Biventricular impairment (lowest quartile left ventricular ejection fraction, <50% and right ventricular ejection fraction, <40%) conveyed an even higher risk of mortality (hazard ratio, 8.0 [95% confidence interval, 2.5-25.1]; P=0.0004). No other CMR or noninvasive measurement besides resting oxygen saturation (hazard ratio, 0.90 [0.83-0.97]/%; P=0.007) was associated with mortality.
CONCLUSIONS
Impaired right, left, or biventricular systolic function derived from baseline CMR and resting oxygen saturation are associated with mortality in adult patients with ES. CMR is a useful noninvasive tool, which may be incorporated in the risk stratification assessment of ES during lifelong follow-up.
Topics: Adult; Cause of Death; Decision Support Techniques; Disease Progression; Echocardiography; Eisenmenger Complex; Exercise Test; Female; Heart Failure; Humans; Hypertension, Pulmonary; Kaplan-Meier Estimate; Longitudinal Studies; Magnetic Resonance Imaging; Male; Middle Aged; Oxygen; Predictive Value of Tests; Prognosis; Proportional Hazards Models; Prospective Studies; Risk Assessment; Risk Factors; Stroke Volume; Time Factors; United Kingdom; Ventricular Dysfunction, Left; Ventricular Dysfunction, Right; Ventricular Function, Left; Ventricular Function, Right
PubMed: 26659374
DOI: 10.1161/CIRCIMAGING.115.003596 -
European Heart Journal May 2016We aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the...
AIMS
We aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the effect of treatment on outcome in the community.
METHODS AND RESULTS
Patients with ES were systematically identified from the German National Register for Congenital Heart Defects. Data on underlying diagnosis, medical therapy, and survival were collected. The impact of DTT on survival was assessed using time-dependant Cox analysis. Overall, 153 ES patients were included (mean age 34.0 ± 13.3 years, 46% females). Of these, 88 (57.5%) were treated with at least one DTT (76.1% Bosentan, 20.5% Sildenafil) while 17.6% were on dual DTT. In addition, 24.8% of patients received digoxin, 10.5% angiotensin-converting enzyme-inhibitors/angiotensin receptor blockers, and 17.6% β-blockers. Moreover, 17.6% of patients were treated with oral anticoagulants, while 23.5% of patients received Aspirin. The survival rate at 1, 5, and 10 years of follow-up was only 92, 75, and 57% in the entire cohort, and was even worse in treatment naive ES patients (survival rate 86, 60, and 34% at 1, 5, and 10 years). Use of DTT was independently associated with a better survival (hazard ratio 0.42, P= 0.015).
CONCLUSION
This study illustrates the alarmingly poor survival prospects of Eisenmenger patients by community-based data even in the current era with advanced DTT and in a country with a wealthy health system. Treatment naive ES patients had especially high mortality rates approaching 60-70% at 10 years of follow-up. Treatment with DTT was associated with better survival.
Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; Anticoagulants; Eisenmenger Complex; Female; Humans; Male; Sildenafil Citrate
PubMed: 26843280
DOI: 10.1093/eurheartj/ehv743 -
Anatolian Journal of Cardiology May 2018To determine the values of microvolt T-wave alternans (MTWA) in children and adolescents with Eisenmenger syndrome (ES) and controls.
OBJECTIVE
To determine the values of microvolt T-wave alternans (MTWA) in children and adolescents with Eisenmenger syndrome (ES) and controls.
METHODS
Thirteen were included in the study. After analyzing the 24-h ECG recordings, MTWA was considered using three leads (V5, V1, and aVF). Right heart catheterization and 6-minute walk test (6-MWD) were applied to the patients and pro-brain natriuretic peptide levels were assessed; echocardiographic parameters were obtained from both the groups and the results were compared.
RESULTS
The MTWA value in lead V5 was 81.08±10.73 µV in the patient group (63.50±18.78 µV in the control group), in lead V1 was 75.00±16.86 µV (73.94±16.77 µV in the control group), and in lead aVF was 73.77±17.81 µV (72.61±16.21 µV in the control group). Comparison of MTWA values between patients and controls revealed that only lead V5 values were statistically different in the ES group. The 6-MWD scores significantly correlated with lead V5. Right atrial volume and right ventricular fractional area change were significantly correlated with lead V1. The Tei index was significantly correlated with lead aVF.
CONCLUSION
The MTWA lead V5 value was significantly higher in children with ES than in controls and was also correlated with decreased exercise tolerance.
Topics: Adolescent; Cardiac Catheterization; Case-Control Studies; Child; Child, Preschool; Echocardiography; Eisenmenger Complex; Electrocardiography; Exercise Test; Female; Heart Conduction System; Humans; Male; Natriuretic Peptide, Brain; Peptide Fragments; Young Adult
PubMed: 29638226
DOI: 10.14744/AnatolJCardiol.2018.60487 -
Circulation Dec 2015Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD...
BACKGROUND
Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.
METHODS AND RESULTS
We included 6969 adult patients (age 29.9 ± 15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.
CONCLUSIONS
ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cause of Death; Cohort Studies; Female; Follow-Up Studies; Heart Defects, Congenital; Humans; Male; Middle Aged; Retrospective Studies; Survival Rate; Tertiary Care Centers; Young Adult
PubMed: 26369353
DOI: 10.1161/CIRCULATIONAHA.115.017202 -
The American Journal of Case Reports Mar 2022BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal...
BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal extramedullary and non-hepatosplenic extramedullary hematopoiesis a 43-year-old man with a history of congenital Eisenmenger syndrome. CASE REPORT We present the case of a 43-year-old patient with complex heart disease and full-blown Eisenmenger syndrome associated with ventricular septal defect, bicuspid right ventricle, and pulmonary hypertension. In July 2020, the patient began to report neurological symptoms in the form of lower-limb numbness and weakness of lower-limb strength, with additional increasing lower-limb edema. A CT scan of the abdomen and pelvis revealed a 63×102×103 mm soft-tissue mass in the pelvis, located behind the urinary bladder. Due to the suspicion of proliferative disease, mainly of the lymphatic system, a diagnostic trephine biopsy was performed first, but no tumor cell infiltration was found. Then, the patient was qualified for diagnostic surgery. During the operation, tumor sections were harvested. Histopathological examination of the tissue sections showed extramedullary hematopoiesis in the tumor lining. Hydroxycarbamide was used as first-line treatment. However, it was not effective in controlling clinical symptoms. Therefore, the patient was qualified for radiotherapy as a second-line palliative treatment. CONCLUSIONS This report presents a patient with cyanotic heart disease and extramedullary and non-hepatosplenic hematopoiesis presenting as masses that mimicked malignancy. In this case, palliative radiotherapy effectively reduced the symptoms due to the size of the mass lesion.
Topics: Adult; Eisenmenger Complex; Hematologic Diseases; Hematopoiesis, Extramedullary; Humans; Liver; Male; Tomography, X-Ray Computed
PubMed: 35332114
DOI: 10.12659/AJCR.935141 -
Cureus Mar 2024This case emphasizes the complexity of Prader-Willi syndrome (PWS), the need for a collaborative approach from specialists, and a closer look at the various...
This case emphasizes the complexity of Prader-Willi syndrome (PWS), the need for a collaborative approach from specialists, and a closer look at the various cardiovascular complexities associated with this syndrome. While current treatments focus on managing symptoms, ongoing genetic research offers hope for more favorable outcomes. Further studies are crucial to gauge the effectiveness of these treatments for PWS patients. We detail a patient with a complex medical history of PWS, further complicated by congenital heart disease with Eisenmenger's syndrome, diabetes mellitus, pulmonary hypertension, venous insufficiency, hypothyroidism, and hyperlipidemia. Reported in this study is a compilation of clinical data as well as suggestions from several medical specialists in applying a multifaceted approach to treatment, significantly emphasizing the need for interdisciplinary care and management of patients experiencing a combination of various medical issues with an emphasis on cardiovascular complications.
PubMed: 38646247
DOI: 10.7759/cureus.56591 -
Annals of Cardiac Anaesthesia 2020Successful management of a pregnant patient with complex congenital heart disease is a challenge for anesthesiologists, requiring thorough knowledge of the impact of...
Successful management of a pregnant patient with complex congenital heart disease is a challenge for anesthesiologists, requiring thorough knowledge of the impact of pregnancy on the cardiac lesion. Hearing and speech impaired patients pose a barrier to effective communication between the patient and the doctors, thus increasing the anxiety and risk of complications. Here, we present a case of a hearing and speech impaired woman with the rare and dangerous Eisenmenger's syndrome, presenting for an emergency cesarean section (CS).
Topics: Adult; Anesthesia, Epidural; Anesthesia, Obstetrical; Cesarean Section; Dyspnea; Eisenmenger Complex; Female; Heart Murmurs; Humans; Pregnancy; Pregnancy Complications, Cardiovascular
PubMed: 31929258
DOI: 10.4103/aca.ACA_175_18